Parents' expectations regarding case management for rare diseases in Switzerland: mixed-method findings from an online survey.

AIMS OF THE STUDY: This pilot study aims to enhance understanding by examining parents' specific views on the requirements, content and objectives of case management and advanced care coordination for children with rare diseases during childhood. The findings of this study are expected to offer valuable insights and recommendations for existing and future initiatives in clinical practice and research, with the goal of improving the comprehensive, child-centred and family-orientated approach to case management. METHODS: This pilot study is part of an ongoing prospective study (SPACE), involving parents and families from various networks in Switzerland. Participants were parents recruited from the Children with Rare Diseases (KMSK) network consisting of families with children with rare diseases. The survey questionnaire covered demographic information; expectations and perceived need for case management; assessment of their quality of life and their child's suffering; and evaluation of interprofessional and interdisciplinary communication. Qualitative data from free-response answers were analysed using Mayring's content analysis and descriptive statistics were used to analyse quantitative data from Likert-scale questions. RESULTS: The study included 108 respondent families from among the 775 in the KMSK, a 14% response rate. The age of their children ranged from 0.4 to 24 years (mean: 8) and their level of suffering in the past six months varied, with 31.5% indicating intense or very intense suffering. In terms of case management, 15.8% of families reported access while 32.4% expressed a need but did not have access to it. The study identified three categories of parental expectations regarding case management, emphasising the importance of interprofessional collaboration, effective communication and comprehensive support. CONCLUSIONS: The findings shed light on the high need for case management support with a current undersupply in Switzerland and an association with reduced parental quality of life, highlighting the necessity for diverse support and assistance to effectively manage the challenges faced by families with children with rare diseases.

Between hope and disillusionment: ECMO seen through the lens of nurses working in a neonatal and paediatric intensive care unit.

BACKGROUND: Using extracorporeal membrane oxygenation (ECMO) in paediatric and neonatal intensive care units (PICU/NICU) creates ethical challenges and carries a high risk for moral distress, burn out and team conflicts. AIM: The study aimed to gain a more comprehensive understanding of the underlying factors affecting moral distress when using ECMO for infants and children by examining the attitudes of ECMO nurses. METHODS: Four focus groups discussions were conducted with 21 critical care nurses working in a Swiss University Children's Hospital. Purposive sampling was adopted to identify research participants. The data were analysed using reflexive thematic analysis. RESULTS: Unlike "miracle machine" stories in online media reports, specialized nurses working in PICU/NICU expressed both their hopes and fears towards this technology. Their accounts also contained references to events and factors that triggered experiences of moral distress: the unspeakable nature of the death of a child or infant; the seemingly lack of honest and transparent communication with parents; the apparent loss of situational awareness among doctors; the perceived lack of recognition for the role of nurses and the variability in end-of-life decision-making; the length of time it takes doctors to take important treatment decisions; and the resource intensity of an ECMO treatment. CONCLUSION: The creation of a multidisciplinary moral community with transparent information among all involved health care professionals and the definition of clear treatment goals as well as the implementation of paediatric palliative care for all paediatric ECMO patients should become a priority if we want to alleviate situations of moral distress. RELEVANCE FOR CLINICAL PRACTICE: The creation of a multidisciplinary moral community, clear treatment goals and the implementation of palliative care for all paediatric ECMO patients are crucial to alleviate situations of moral distress for nurses, and thus to improve provider well-being and the quality of patient care in PICU/NICU.

Pediatric Shared Decision-Making for Simple and Complex Decisions: Findings From a Delphi Panel.

OBJECTIVE: To develop recommendations for pediatric shared decision-making (SDM). METHODS: We conducted a Delphi method study from 2020 to 2021 with an international panel (n = 21) of clinicians, researchers, and parents with expertise in pediatric SDM. We conducted semistructured interviews to identify the key processes of pediatric SDM. We coded the interviews using content analysis and developed a questionnaire on the potential processes of pediatric SDM. Using a Likert scale, panelists evaluated each process twice, once for simple decisions and once for complex decisions. Panelists were provided with a summary of the results and evaluated each process again. The processes that were agreed on for simple and complex decisions were reported as "fundamental processes." The processes that were agreed on for complex decisions were reported as "additional processes." RESULTS: A total of 79 recommendations were developed, including 29 fundamental processes and 14 additional processes for complex decisions. A recurring theme was the importance of personalizing the decision-making process. For example, the panel recommended that physicians should assess the family and child's desired roles in the decision-making process, assess their desired level of directiveness, and elicit and clarify their values, preferences, and goals. The panel also disagreed on several subprocesses, such as how to determine the child's role and the appropriate level of directiveness. CONCLUSIONS: An international expert panel developed recommendations for pediatric SDM for both simple and complex decisions. The recommendations highlight the importance of personalizing the decision-making process.

[Shared Decision-Making in Pediatrics - a Collaborative Process within the Therapeutic Triangle between Parents, the Child and Professionals]. / Shared Decision-Making in der Pädiatrie.

Shared Decision-Making in Pediatrics - a Collaborative Process within the Therapeutic Triangle between Parents, the Child and Professionals Abstract. Shared Decision-Making in pediatrics is a competence and a process to make objective facts and subjective perspectives within the therapeutic triangle of parents, child, and professionals visible, understandable, and manageable. The connection of subjective and objective aspects takes place in a spectrum ranging from the interpretation of family values by professionals to the independent assessment of information by the child and/or family. The specificity of decision making in pediatric and adolescent medicine lies in the combination of best interests and shared decision making and the clarification of well-founded threshold of harm.

No wrong decisions in an all-wrong situation. A qualitative study on the lived experiences of families of children with diffuse intrinsic pontine glioma.

BACKGROUND: Diffuse intrinsic pontine glioma (DIPG) is a rare, but lethal pediatric brain tumor with a median survival of less than 1 year. Existing treatment may prolong life and control symptoms, but may cause toxicity and side effects. In order to improve child- and family-centered care, we aimed to better understand the treatment decision-making experiences of parents, as studies on this topic are currently lacking. PROCEDURE: The data for this study came from 24 semistructured interviews with parents whose children were diagnosed with DIPG in two children's hospitals in Switzerland and died between 2000 and 2016. Analysis of the dataset was done using reflexive thematic analysis. RESULTS: For most parents, the decision for or against treatment was relatively straightforward given the fatality of the tumor and the absence of treatment protocols. Most of them had no regrets about their decision for or against treatment. The most distressing factor for them was observing their child's gradual loss of independence and informing them about the inescapability of death. To counter this powerlessness, many parents opted for complementary or alternative medicine in order to "do something." Many parents reported psychological problems in the aftermath of their child's death and coping strategies between mothers and fathers often differed. CONCLUSION: The challenges of DIPG are unique and explain why parental and shared decision-making is different in DIPG compared to other cancer diagnoses. Considering that treatment decisions shape parents' grief trajectory, clinicians should reassure parents by framing treatment decisions in terms of family's deeply held values and goals.

[Pediatric Palliative Care - When the End Comes with the Beginning]. / Pädiatrische Palliative Care ­ wenn das Ende mit dem Anfang kommt.

Pediatric Palliative Care - When the End Comes with the Beginning Abstract. Palliative care is not usually associated with childhood and adolescence. Nevertheless, in German-speaking Europe alone, more than 50 000 children live with life-shortening illnesses, and about 5000 children die every year. Palliative care for children and adolescents (PPC) is an attitude that must not be forced on anyone, but must be lived; and, in the case of complex-chronic diseases often over many years. A successful and sustainable implementation of PPC is therefore based on an appreciative collaboration between different teams and the affected families, true to the quadriga presented here of comprehensive symptom control with curiosity, patience, activity and humility. Necessary basics such as the care plan, interprofessional home visits and the round table, plus 24-hour accessibility, are here presented, as well as a triage technique and the advanced care management.

Early Genital Surgery in Disorders/Differences of Sex Development: Patients' Perspectives.

Controversy continues over a proposed moratorium on elective genital surgery in childhood for disorders/differences of sex development (DSD). Empirical evidence on patient preference is needed to inform decision-making. We conducted a multicentre survey by cross-sectional questionnaire in 14 specialized clinics in six European countries. The sample comprised 459 individuals (≥ 16 years) with a DSD diagnosis, including individuals with congenital adrenal hyperplasia (CAH) (n = 192), XY DSD with prenatal androgen effect (A) (n = 150), and without (nA) (n = 117). Main outcome measures were level of agreement with given statements regarding genital surgery, including clitoris reduction, vaginoplasty, and hypospadias repair. A total of 66% of individuals with CAH and 60% of those with XY DSD-A thought that infancy or childhood were the appropriate age for genital surgery. Females with XY DSD were divided on this issue and tended to prefer vaginoplasty at a later age (XY DSD-A 39%, XY DSD-nA 32%). A total of 47% of males preferred early hypospadias surgery. Only 12% (CAH), 11% (XY DSD-A), and 21% (XY DSD-nA) thought they would have been better off without any surgery in childhood or adolescence. Individuals who had early genital surgery were more likely to approve of it. Outcome data failed to support a general moratorium on early elective genital surgery. Participant perspectives varied considerably by diagnostic category, gender, history of surgery, and contact with support groups. Case-by-case decision-making is better suited to grasping the ethical complexity of the issues at stake.Trial registration: German Clinical Trials Register DRKS00006072.

Specialized Pediatric Palliative Care in Neonates with Life-Limiting Illness: A Systematic Review.

OBJECTIVE: The aim of this study is to assess the impact of specialized pediatric palliative care (PPC) on neonates with life-limiting conditions compared to standard care. STUDY DESIGN: MEDLINE, PsycINFO, Cochrane Central Register of Controlled Trials, Web of Science, CINAHL, Scopus, and Embase databases were searched from January 2000 to September 2018. Randomized clinical trials, experimental or observational studies, and secondary administrative database analyses published in English, Spanish, French, and German were included. Two independent reviewers extracted data, and used the Newcastle-Ottawa Scale and the Cochrane Risk of Bias Tool for quality analysis. Discrepancies were resolved as a team. RESULTS: From the 37,788 records obtained, only eight articles met the inclusion criteria. A meta-analysis was not possible due to the heterogeneity in how the outcomes were defined; however, a qualitative synthesis of the results was possible; organizing outcomes into eight different categories: psychological, social and spiritual support; communication; location of care; symptom management; bereavement care; predicted versus actual neonatal outcomes; and parental coping, stress, and satisfaction. CONCLUSION: Specialized versus may have an impact on neonates with life-limiting conditions and their families. More studies that evaluate the impact of specialized versus in neonates with sound statistical analysis is warranted.

Combining the best interest standard with shared decision-making in paediatrics-introducing the shared optimum approach based on a qualitative study.

Paediatric decision-making is the art of respecting the interests of child and family with due regard for evidence, values and beliefs, reconciled using two important but potentially conflicting concepts: best interest standard (BIS) and shared decision-making (SD-M). We combine qualitative research, our own data and the normative framework of the United Nations Convention on the Rights of Children (UNCRC) to revisit current theoretical debate on the interrelationship of BIS and SD-M. Three cohorts of child, parent and health care professional interviewees (Ntotal = 47) from Switzerland and the United States considered SD-M an essential part of the BIS. Their responses combined with the UNCRC text to generate a coherent framework which we term the shared optimum approach (SOA) combining BIS and SD-M. The SOA separates different tasks (limiting harm, showing respect, defining choices and implementing plans) into distinct dimensions and steps, based on the principles of participation, provision and protection. The results of our empirical study call into question reductive approaches to the BIS, as well as other stand-alone decision-making concepts such as the harm principle or zone of parental discretion.Conclusion: Our empirical study shows that the BIS includes a well-founded harm threshold combined with contextual information based on SD-M. We propose reconciling BIS and SD-M within the SOA as we believe this will improve paediatric decision-making. What is Known: • Parents have wide discretion in deciding for their child in everyday life, while far-reaching treatment decisions should align with the child's best interest. • Shared decision-making harbours potential conflict between parental authority and a child's best interest. What is New: • The best interest standard should not be used narrowly as a way of saying "Yes" or "No" to a specific action, but rather in a coherent framework and process which we term the shared optimum approach. • By supporting this child-centred and family-oriented process, shared decision-making becomes crucial in implementing the best interest standard.

Correction to: Decision-making at the limit of viability: differing perceptions and opinions between neonatal physicians and nurses.

After publication of our article [1] it was brought to our attention that we did not have permission to reproduce the questionnaire in Additional File 1.

Special Parents for "Special" Children? The Narratives of Health Care Providers and Parents of Intersex Children.

Decisions on the clinical management of children with an intersex condition heavily depend on the kind of information parents receive from healthcare professionals. This paper aims to explore this influence by analyzing the stories of parents and care providers. The presented data come from 19 semi-structured interviews with parents of intersex children and healthcare professionals specializing in intersex conditions in Switzerland and Germany. Arthur Frank's narrative framework was used to analyze the stories and sort out which kind of cultural threads were available to participants to make sense of their experience. Although many healthcare providers discouraged early genitoplasty, they believed that the decision not to operate required unique parenting skills, which few parents possess. This vision was shared by the parents in our sample. Some parents also projected this specialness upon their children, viewing the condition as a special gift, and as a possibility of personal growth.

"Undoing" Capacity: The Capability Approach in Pediatrics.

Unlike adults, children are not granted the assumption of having decision-making capacity because their cognitive capacities are not yet fully developed. Still, child participation is increasingly encouraged within the clinical and research context. The trend towards inclusion has been initiated by the United Nations Convention on the Rights of Children (1989). The openness of the convention, however, might lead to contradictory interpretations. The notion of evolving capacities recalls mainstream developmental psychological theories that view the child as an impaired being and may hamper children's right to participation. This shows that policy measures are not a panacea and that other tools are needed to promote children's involvement in medical practice. For this purpose, the authors of this essay aim to "undo" the traditional, cognitive approach to decision-making capacity and to incorporate the conceptual framework of the capability approach in pediatrics. The capability approach encourages parents and health-care professionals to reflect on how they can foster children's role in decision-making by taking into consideration the socio-familial, cultural, and environmental context in which they live. By acknowledging that children's preferences can differ, the capability approach promotes a patient-centered approach.

Impact of specialized pediatric palliative care programs on communication and decision-making.

OBJECTIVE: To summarize and analyze the impact of specialized pediatric palliative care (SPPC) programs on communication and decision-making for children with life-threatening conditions. METHODS: Our search strategy covered MEDLINE, PsycINFO, Cochrane Central Register of Controlled Trials, Web of Science, CINAHL, Scopus, and Embase through September 2018. RESULTS: We reviewed 13 studies analyzing the impact of SPPC programs on communication and decision-making using a wide range of outcome indicators. Study quality was poor in 58% of included papers. SPPC programs improved communication and decision-making between families and healthcare professionals (HCPs), within and between families, and among HCPs. CONCLUSION: SPPC programs generally support and improve communication and decision-making for children with life-threatening conditions, their families and associated HCPs. Families referred to an SPPC program had more discussions with HCPs on a broad variety of topics. However, data on communication with children, siblings, and other family members was scarce and of poor quality. PRACTICE IMPLICATIONS: More research on SPPC program efficacy is needed from the perspective of the ill child, as well as about barriers to end-of-life discussions and the specific aspects of SPPC programs responsible for improving outcomes.

Indicators Used to Assess the Impact of Specialized Pediatric Palliative Care: A Scoping Review.

BACKGROUND: Specialized pediatric palliative care programs aim to improve quality of life and ease distress of patients and their families across the illness trajectory. These programs require further development, which should be based on how they improve outcomes for patients, families, health care professionals, and the health care system. OBJECTIVE: To identify and compare definitions of indicators used to assess the impact of specialized pediatric palliative care programs. DESIGN: The scoping review protocol was prospectively registered on PROSPERO 2017 (CRD42017074090). DATE SOURCES: MEDLINE, PsycINFO, Cochrane Central Register of Controlled Trials, Web of Science, CINAHL, Scopus, and Embase databases were searched from January 2000 to September 2018. Eligible studies included randomized controlled trials, experimental studies, or observational studies that compared specialized programs with usual care. Studies were excluded if most care recipients were older than 19 years or the article was not available in English, French, German, or Spanish. RESULTS: Forty-six studies were included; one was a randomized controlled trial. We identified 82 different indicators grouped into 14 domains. The most common indicators included the following: location of death, length of stay in hospital, and number of hospital admissions. Only 22 indicators were defined identically in at least 2 studies. Only one study included children's perspectives in assessing indicators. CONCLUSIONS: Many indicators were used to assess program outcomes with little definition consensus across studies. Development of a set of agreed-upon indicators to assess program impact concurrent with family and patient input is essential to advance research and practice in pediatric palliative care.

Correction to: decision-making at the limit of viability: differing perceptions and opinions between neonatal physicians and nurses.

After publication of the original article [1], the corresponding author noticed the given names and family names of the members included in the Swiss Neonatal End-of-Life Study Group were incorrectly reverted.

Decision-making at the limit of viability: differing perceptions and opinions between neonatal physicians and nurses.

BACKGROUND: In the last 20 years, the chances for intact survival for extremely preterm infants have increased in high income countries. Decisions about withholding or withdrawing intensive care remain a major challenge in infants born at the limits of viability. Shared decision-making regarding these fragile infants between health care professionals and parents has become the preferred model today. However, there is an ongoing ethical debate on how decisions regarding life-sustaining treatment should be reached and who should have the final word when health care professionals and parents do not agree. We designed a survey among neonatologists and neonatal nurses to analyze practices, difficulties and parental involvement in end-of-life decisions for extremely preterm infants. METHODS: All 552 physicians and nurses with at least 12 months work experience in level III neonatal intensive care units (NICU) in Switzerland were invited to participate in an online survey with 50 questions. Differences between neonatologists and NICU nurses and between language regions were explored. RESULTS: Ninety six of 121 (79%) physicians and 302 of 431(70%) nurses completed the online questionnaire. The following difficulties with end-of-life decision-making were reported more frequently by nurses than physicians: insufficient time for decision-making, legal constraints and lack of consistent unit policies. Nurses also mentioned a lack of solidarity in our society and shortage of services for disabled more often than physicians. In the context of limiting intensive care in selected circumstances, nurses considered withholding tube feedings and respiratory support less acceptable than physicians. Nurses were more reluctant to give parents full authority to decide on the course of action for their infant. In contrast to professional category (nurse or physician), language region, professional experience and religion had little influence if any on the answers given. CONCLUSIONS: Physicians and nurses differ in many aspects of how and by whom end-of-life decisions should be made in extremely preterm infants. The divergencies between nurses and physicians may be due to differences in ethics education, varying focus in patient care and direct exposure to the patients. Acknowledging these differences is important to avoid potential conflicts within the neonatal team but also with parents in the process of end-of-life decision-making in preterm infants born at the limits of viability.

Sources of distress for physicians and nurses working in Swiss neonatal intensive care units.

BACKGROUND: Medical personnel working in intensive care often face difficult ethical dilemmas. These may represent important sources of distress and may lead to a diminished self-perceived quality of care and eventually to burnout. AIMS OF THE STUDY: The aim of this study was to identify work-related sources of distress and to assess symptoms of burnout among physicians and nurses working in Swiss neonatal intensive care units (NICUs). METHODS: In summer 2015, we conducted an anonymous online survey comprising 140 questions about difficult ethical decisions concerning extremely preterm infants. Of these 140 questions, 12 questions related to sources of distress and 10 to burnout. All physicians and nurses (n = 552) working in the nine NICUs in Switzerland were invited to participate. RESULTS: The response rate was 72% (398). The aspects of work most commonly identified as sources of distress were: lack of regular staff meetings, lack of time for routine discussion of difficult cases, lack of psychological support for the NICU staff and families, and missing transmission of important information within the caregiver team. Differences between physicians' and nurses' perceptions became apparent: for example, nurses were more dissatisfied with the quality of the decision-making process. Different perceptions were also noted between staff in the German- and French- speaking parts of Switzerland: for example, respondents from the French part rated lack of regular staff meetings as being more problematic. On the other hand, personnel in the French part were more satisfied with their accomplishments in the job. On average, low levels of burnout symptoms were revealed, and only 6% of respondents answered that the work-related burden often affected their private life. CONCLUSIONS: Perceived sources of distress in Swiss NICUs were similar to those in ICU studies. Despite rare symptoms of burnout, communication measures such as regular staff meetings and psychological support to prevent distress were clearly requested.

Exploring societal solidarity in the context of extreme prematurity.

QUESTION: Extreme prematurity can result in long-term disabilities. Its impact on society is often not taken into account and deemed controversial. Our study examined attitudes of the Swiss population regarding extreme prematurity and people's perspectives regarding the question of solidarity with disabled people. METHODS: We conducted a nationwide representative anonymous telephone survey with 1210 Swiss residents aged 18 years or older. We asked how people estimate their own personal solidarity, the solidarity of their social environment and the solidarity across the country with disabled persons. Spearman's correlation calculations were used to assess if a correlation exists between solidarity and setting financial limits to intensive care and between solidarity and withholding neonatal intensive care. RESULTS: According to 36.0% of the respondents intensive medical care should not be withheld from extremely preterm infants, even if their chances for an acceptable quality of life were poor. For 28.8%, intensive care should be withheld from these infants, and 26.9% held an intermediate position depending on the situation. A total of 31.5% were against setting a financial limit to treatment of extremely preterm newborns with an uncertain future quality of life, 34.2% were in favour and 26.9% were deliberating. A majority (88.8%) considered their solidarity toward disabled people as substantial; the solidarity of their personal environment and of the society at large was estimated as high by 79.1% and 48.6%, respectively. CONCLUSIONS: The Swiss population expressed a high level of solidarity which may alleviate some pressure on parents and health care providers in the decision-making process in neonatal intensive care units. In addition, there was no relationship between solidarity and people's willingness to pay for the care or withholding treatment of extremely preterm babies.

Attitudes towards decisions about extremely premature infants differed between Swiss linguistic regions in population-based study.

AIM: Studies have provided insights into the different attitudes and values of healthcare professionals and parents towards extreme prematurity. This study explored societal attitudes and values in Switzerland with regard to this patient group. METHODS: A nationwide trilingual telephone survey was conducted in the French-, German- and Italian-speaking regions of Switzerland to explore the general population's attitudes and values with regard to extreme prematurity. Swiss residents of 18 years or older were recruited from the official telephone registry using quota sampling and a logistic regression model assessed the influence of socio-demographic factors on end-of-life decision-making. RESULTS: Of the 5112 people contacted, 1210 (23.7%) participated. Of these 5% were the parents of a premature infant and 26% knew parents with a premature infant. Most participants (77.8%) highlighted their strong preference for shared decision-making, and 64.6% said that if there was dissent then the parents should have the final word. Overall, our logistic regression model showed that regional differences were the most significant factors influencing decision-making. CONCLUSION: The majority of the Swiss population clearly favoured shared decision-making. The context of sociocultural demographics, especially the linguistic region in which the decision-making took place, strongly influenced attitudes towards extreme prematurity and decision-making.